PATHOPHYSIOLOGICAL MECHANISMS IN TYPE I VWD

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This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. This project is a study of the mechanisms that result in the clinical diagnosis of Type 1 VWD. While most individuals have a reduced synthesis of normal protein, there are other individuals that produce a VWF with an altered survival causing a more rapid clearance and a decrease in plasma concentration of the VWF protein. Since VWF multimerization can be accomplished prior to VWF storage, some individuals may also have a decreased ability to store VWF and thus, have a decreased clinical response to stress and pharmacologic agents such as DDAVP.